In females with congenital adrenal hyperplasia, 21-hydroxylase deficiency without salt loss is usually recognized in the neonate because of ambiguous genitalia (ie, clitoromegaly with or without labial fusion), or at a later age because of progressive virilization and/or accelerated linear growth. We are reporting the second case of posterior labial fusion without clitoromegaly as the presenting sign of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Here, as in the prior case of Wolff et al,1 the absence of clitoromegaly postponed recognition of the patient's problem.


C.V. was born at term to a 25-year old, gravida 2, para 1, Mexican-American woman after a normal pregnancy; only vitamins were taken during the pregnancy.

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